Split notochord syndrome with neuroenteric fistula: A case report

Introduction

Split notochord syndrome (SNS) is a rare congenital anomaly characterized by malformations in the spine, central nervous system and gastrointestinal tract. This paper presents a case of SNS accompanied by a neuroenteric fistula (NEF).

Case presentation

A 7-day-old male neonate was admitted to our NICU for the evaluation and management of a lower back swelling with meconium extrusion, following an emergency cesarean section at 38 + 3 weeks gestational age due to meconium-stained amniotic fluid. The mother had a normal antenatal care history without reported fetal abnormalities. The patient had a noticeable lower back mass with active meconium oozing, accompanied by flaccid paralysis of the lower extremities. Blood work revealed elevated inflammatory markers and normal renal function. Abdominal CT scan and MRI identified a complex spinal malformation indicative of SNS, characterized by a thoracolumbar cystic mass, spinal bifida, non-fusion of lumbar vertebral bodies, a neuroenteric fistula, and Chiari II malformation, in addition to a right inguinal hernia and hydronephrosis. The patient underwent a laparotomy during which intestinal malrotation and a colonic duplication were also found. We did a NEF separation, a double barrel sigmoid colostomy, and a Ladd's procedure. By the 23rd day of life, the patient developed fever, tachypnea, tachycardia, and had purulent discharge from the surgical site. Despite the antibiotic treatment, the patient's condition deteriorated. He died on day-of-life 28 from overwhelming sepsis.

Conclusion

Our case highlights the complexity of patients with SNS and the need for enhanced surveillance and prompt responses in patients with this rare congenital malformation.